Xeroderma pigmentosum is one of the group of genodermatoses and is characterized as being an autosomal recessive defect resulting in marked hypersensitivity to ultraviolet radiation of the skin with early development of intense sunburn reaction, diffuse pigmentary changes is the sun exposed skin, the development of benign and ultimately malignant tumors of the skin, severe ocular and neurologic abnormalities, and frequently death from metastatic tumor in the first 2 decades of the life. This disease is very rare in Korea, so that a few cases have been reported.
Authors experienced a case of 31 year male, who visited Chung-Ang University Hospital complaining of multiple skin masses in the face, ectropion of left lower eyelid, caused by scar contracture which had resulted from cryosurgery as treatment for basal cell epithelioma in the face in 1977.
He had been treated for malignant melanoma and basal cell epithelioma on the face by excision in our department(1985). But, thereafter, he had not been treated again until he visited to our department(1987).
We performed excisional biopsy for the skin masses and surgical correction of ectropion of left lower eyelid. The histopathologic findings of skin masses revealed various types of basal cell epithelioma(Adenoid, Metatypical, Pigmented type). During the follow-up study for 6 months, there has been no evidence of recurrence.
We hereby represent a case of xeroderma pigmentosum developing malignant melanoma and various types of basal cell epithelioma in the same patient, and review of various references of xeroderma pigmentosum.
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